Background: Pregnancy is a relatively infrequent event in women with Systemic Sclerosis (SSc) and may be characterized by an increased risk of maternal and foetal complications. Considering the heterogenous nature of SSc in terms of type of organ involvement and severity, counselling and management of pregnancy are particularly challenging and evolving over time. Objectives: The purpose of the study is to evaluate pregnancy outcome in a cohort of SSc patients followed in a dedicated multidisciplinary clinic with a team of Rheumatologists, Gynaecologists-Obstetrics and Neonatologists. Methods: Retrospective analysis of pregnancies in patients with SSc (2013 ACR/EULAR criteria) or undifferentiated connective tissue disease "at risk of SSc" (UCTD-SSc risk, according to Valentini 2020 [1]) prospectively followed in our Pregnancy Clinic (2008-2023). Results: We followed-up 44 pregnancies (4 ongoing) in 33 women: 23 SSc and 10 UCTD-SSc risk (Table 1). Median age at conception was 34 (31-37) years and median disease duration was 62 (0-133) months for SSc women (in 2 patients <2 years). In 35/44 cases (79%) a pre-conception counselling was provided, while the patient was referred during the first trimester in 9 cases (21%). In 91% of the pregnancies low dose aspirin (LDA) was prescribed to prevent hypertensive gestational disorders and it was started either during preconception period or at positive pregnancy test. None of the patients experienced worsening of disease manifestations during pregnancy, except for esophageal reflux which was easily managed with anti-acids. After delivery, one patient with severe interstitial lung disease (ILD) who did not receive a preconception counselling had respiratory insufficiency and congestive heart failure. Another patient with limited cutaneous SSc and positive anti-Topo I, whose disease onset was <2 years, rapidly developed diffused cutaneous SSc and ILD. A patient with previous scleroderma renal crisis (SRC) had preeclampsia (PE) at 36 gestational week (GW) in her first pregnancy and gestational hypertension during the second one, both hesitated in live births at term. Regarding pregnancy outcomes (Table 2): excluding 4 ongoing pregnancies, the remaining 40 ended in 4 miscarriages prior to 10 GW, 1 intrauterine at 12 GW and 35 live births (36 babies, 1 twin pregnancy; 1 perinatal death was recorded after pPROM due to amniocentesis at 24w). Preterm delivery (<37w) was recorded in 8 cases (23%), including 5 severe preterm <34w (14%), 3 (9%) of which were due to PE. Conclusion: The retrospective analysis of an historical cohort of SSc pregnancies highlighted an increased frequency of preterm deliveries and hypertensive disorders as compared to Italian general obstetric population (25% vs 6% [2] and 13% vs 5% [3], respectively), despite prophylaxis with LDA in 90% of patients. A multidisciplinary dedicated setting is crucial to provide a preconception counselling and tailored management during pregnancy to reduce the rate of adverse outcomes. Patients with severe organ involvement or recent disease onset should deserve special attention. REFERENCES: [1] Valentini G, Pope JE. Autoimmun Rev. 2020. [2] Abalos E, et al. Eur J Obstet Gynecol Reprod Biol. 2013. [3] Certificato di assistenza al parto (CeDAP). Analisi dell'evento nascita - Anno 2022; https://www.salute.gov.it, last access 17 11 2023. Acknowledgements: Authors would like to thank GILS (Gruppo Italiano Lotta Sclerodermia) for substantially supporting this project. Disclosure of Interests: None declared.
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